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Introduction: IgA vasculitis is more commonly seen in the pediatric population than in adults. Rarely IgA vasculitis is associated with malignancy, most commonly solid tumor malignancies, although there are case reports of association with hematologic malignancies. We report a case of large B-cell lymphoma mimicking IgA vasculitis in a 33-year-old immunosuppressed male with a prior history of IgA vasculitis. Case Description/Methods: A 33-year-old Caucasian male post renal transplant from reflux nephropathy on chronic immunosuppression was hospitalized for postprandial epigastric abdominal pain, nausea, vomiting and diarrhea. Two years prior, he was admitted for the same symptoms, palpable purpura of the lower extremities and elevated serum IgA. Enteroscopy had shown duodenal and jejunal ulceration with biopsies staining positive for IgA, confirming IgA vasculitis. He had complete resolution with a steroid taper. His current presentation had resulted in multiple hospital admissions, but empiric trial of steroids failed to alleviate symptoms. Vitals were normal and exam was notable for epigastric tenderness. Labs were notable for WBC 19.00 x103/cmm with normal differential, hemoglobin 9.2 gm/dL (prior 11.0 gm/dL), CRP 20.7 mg/L, serum creatinine 2.7 mg/dL (prior 1.5 mg/dL), and urinalysis with proteinuria, sterile pyuria, and hematuria. CTA abdomen/pelvis revealed thickening of the duodenum with shotty mesenteric lymph nodes without ischemia. Enteroscopy revealed an erythematous duodenum and jejunum (figure A). Jejunal biopsy (figure B) revealed CD20 positive cells consistent with DLCBL (figure C). He was seen by oncology and treated with R-CHOP but later unfortunately expired due to COVID-19 complications. Discussion(s): Non small cell lung cancer and renal cell carcinoma are most commonly associated with IgA vasculitis. It may also be seen in both Hodgkin and Non-Hodgkin lymphomas in adult patients. If IgA vasculitis occurs after a malignancy is diagnosed, it may indicate that metastasis has occurred. Malignancy associated IgA vasculitis is more likely to have an incomplete response to steroids and requires treatment of the underlying malignancy to achieve remission. Our case illustrates posterior probability error and premature closure cognitive biases. We should consider alternative diagnoses rather than anchor on prior diagnoses even when presentations are similar. Our case also highlights the importance of considering occult malignancy in adults with diagnosis of IgA vasculitis.
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Concurrent with the administration of vaccines for the novel coronavirus (COVID-19), there have been many reported adverse effects, and many of them anticipated as with any vaccine administration. This case report, however, focuses on a patient who, shortly after receiving the first dose of the Moderna COVID-19 vaccine, developed a pruritic maculopapular rash with typical distribution and clinical characteristics, along with high levels of immunoglobulin A (IgA), consistent with IgA vasculitis, formerly known as Henoch-Schonlein purpura. Although there are reported cases of IgA vasculitis after different vaccine administrations, to our knowledge, there are no reports of development of this condition after COVID-19 vaccination. The patient did not have any other triggering events or factors that could be attributed to the development of this pathology. This case describes the development of IgA vasculitis after the COVID-19 vaccination. Copyright © Wolters Kluwer Health, Inc. All rights reserved.
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Case report Introduction: The COVID-19 pandemic has generated an inexorable cost to governments and a high mortality in general terms, the vaccination process is the only effective strategy to reduce morbidity and mortality in general, however, such administration is not without risks. Case presentation: Female patient of the seventh decade who is vaccinated against COVID-19 and 4 days after the second dose of vaccine against COVID-19 begins to present purpuric lesions that begin in the lower limbs and progressively ascend to the trunk associated with intense arthralgias, abdominal pain and nausea, presents macroscopic rectal bleeding, is admitted finding platelet counts lower than 10 thousand platelets, the diagnosis of a Henoch Scholein purpura associated with a vaccine against COVID-19 was confirmed no requiring specific immunosuppressive therapy with resolution at 21 days. The triggering mechanisms are being studied and described as similar to immunological thrombocytopenia due to heparins. Antibodies against platelet factor 4 have been reported so far. The test known as PIFPA (PF4-induced flow cytometry-based platelet activation) is a cytometric test with high sensitivity and specificity, only available in specialized laboratories, being described for the moment as the only test that allows the detection of immunological thrombocytopenic phenomena secondary to vaccination against COVID-19. Discussion(s): The present case constitutes the appearance of a Henoch Scholein Purpura, a rare pathology with a wide repertoire of triggering events, with infections and vaccine reactions among the main triggers. The present vaccine reaction is rare, being the specific anecdotal, until now there are very few descriptions in the literature about this type of vaccine reaction, which is why it is decided to publish it, mentioning the most recent scientific evidence available on this area. It is noteworthy that these are rare reactions that should not make us underestimate continuing with vaccination campaigns as the best strategy to prevent the advance of this pandemic.
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Introduction: There have been some reports on flare-ups of kidney diseases following COVID-19 vaccines such as IgA nephropathy and minimal change disease. However, there have been few reports on those of IgA vasculitis following the vaccines yet. We report a case of IgA vasculitis with a flare-up of gross hematuria and lower-limb purpura following Moderna COVID-19 vaccines. Method(s): The patient is a 16-year-old female with no previous history of abnormal results of urinalyses before April in 2021. She had developed microscopic hematuria, proteinuria and purpura on both of her lower limbs that emerged and then disappeared repeatedly since then. She received Moderna COVID-19 vaccines in August and September in 2021, both of which were followed by gross hematuria lasting for around 10 days. The lower-limb purpura reemerged at the same time as the hematuria. Microscopic hematuria of around 30-49 RBC/HPF, glomerular hematuria of moderate degree and urine protein-to-creatinine ratio (UPCR) of around 0.8 g/gCr had continuously been detected. Skin and kidney biopsies were performed in December in 2021 and in February in 2022 respectively. Result(s): The skin tissue showed formation of leukocytoclastic vasculitis, and the kidney tissue showed that of cellular and fibrocellular crescents and endocapillary hypercellularity. Immunofluorescence staining of both tissues showed deposition of galactose-deficient IgA1(Gd-IgA1) and C3, and she was diagnosed as IgA vasculitis. She received steroid pulse therapy followed by tonsillectomy. The lower-limb purpura has disappeared after she received three courses of the steroid pulse therapy, but microscopic hematuria and UPCR of around 0.8 g/gCr have still continued. Conclusion(s): IgA vasculitis is leukocytoclastic vasculitis characterized by deposition of Gd-IgA1 on microvessel walls in skin and on glomerular capillaries in kidneys. The detailed mechanism of IgA vasculitis has not been fully elucidated yet. Gross hematuria following an upper respiratory infection is considered as a characteristic clinical symptom of IgA vasculitis as well as IgA nephropathy. Post-vaccination gross hematuria of patients with IgA nephropathy has been reported, and it is believed that innate immunity is related to its mechanism. Moderna COVID-19 vaccines, which the patient received, are mRNA vaccines. We estimate that exposure to the mRNA vaccine triggered excess glomerular deposition of Gd-IgA1-containing immune complexes and subsequent gross hematuria by overactivation of innate immunity such as Toll-like receptors that detect RNA. This case suggests that such immune activation by a mRNA vaccine might be related not only to the mechanism of IgA nephropathy but also to that of IgA vasculitis. No conflict of interestCopyright © 2023
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IgA vasculitis nephritis (Schonlein-Henoch purpura nephritis) is an autoimmune circumstance characterized by palpable purpura involving the lower limbs, arthralgia, abdominal pain and kidney involvement. It is possible that a cytokine storm following coronavirus disease 2019 (COVID-19) could lead to an immunological dysregulation responsible for IgA vasculitis nephritis in these cases. Reactivation or first onset of IgA vasculitis nephritis is uncommon;however, there have been increasing reports of this disease, as a complication of COVID-19 vaccination. It is possible that COVID-19 mRNA vaccination may trigger several auto-inflammatory and autoimmune cascades. Previous research has shown that Toll-like receptors play a role in the development of IgA vasculitis nephritis. Following injection of a COVID-19 mRNA vaccine, the uptake of double-stranded RNA by-products will trigger Toll-like receptors, leading to a series of intracellular cascades starting an innate immunity-driven process of cell-mediated and humoral-mediated immunity.Copyright © 2023 The Author(s);Published by Society of Diabetic Nephropathy Prevention. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Immunoglobulin A (IgA) nephropathy is the most common type of glomerulonephritis worldwide characterized by excessive serum levels of glycosylated which triggers the generation of glycan-specific IgG and IgA autoantibodies. This pathological condition results in the formation of circulatory IgA immune complexes, which are essential for the development of glomerular inflammation, especially IgA nephropathy. The serum galactosylated IgA1, IgG, and IgA autoantibodies are suggested as the biomarkers of IgA nephropathy since IgA antibodies are early markers for disease activity too. Serum IgA antibodies emerged as the early COVID-19-specific antibody response about two days after initial symptoms of COVID-19 in comparison with IgG and IgM antibody concentrations, which appeared after five days. IgA nephropathy is frequently presented as microscopic or macroscopic hematuria and proteinuria with a male predominance. COVID-19 infection can include several organs aside from the lungs, such as kidneys through different mechanisms. It is demonstrated in most cases that short-lasting symptoms such as gross hematuria resolve either spontaneously or following a short course of steroids. This review summarized the reported cases of relapses or denovo reported cases of relapses or de-novo IgA nephropathy and IgA vasculitis following COVID-19 vaccination.Copyright © 2023 The Author(s);Published by Society of Diabetic Nephropathy Prevention. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Introduction: The effective control of coronavirus disease 2019 (COVID-19) can be achieved by implementing a global vaccination strategy. After millions of mRNA vaccines targeting severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) have been administered worldwide, several reports have shown the cases with gross hematuria (GH) following the mRNA vaccine against SARS-CoV2 in patients with glomerulonephritis, especially immunoglobulin A nephropathy (IgAN). A total of 22 articles including 36 cases of GH after COVID-19 vaccination as on July 31, 2022, were found in PubMed and Google Scholar databases. The most cases which had performed kidney biopsy were IgAN or IgA vasculitis. So, it suggested that GH after COVID-19 vaccination is rerated IgAN. Although there are many reported cases of IgAN after COVID-19 vaccination, the detailed clinical characteristics and outcome have not determined in these patients. Moreover, it remains unclear whether COVID-19 vaccination causes the new onset of nephritis or exacerbates pre-existing nephritis. To address this, herein, we conducted a prospective cohort study over a six-month period. Method(s): We analyzed 82 patients who presented with GH after COVID-19 vaccination and conducted a 6-month observational study. Patients, 18 years or older, who presented to the hospital with GH after COVID-19 vaccination were recruited. All the patients visited either Juntendo University Hospital or Juntendo University Urayasu Hospital between May 11, 2021, and July 31, 2022. Result(s): During the study period, a total of 82 individuals who presented with GH after COVID-19 vaccination were enrolled. The median age of the patients was 38 years;58 cases (70.7%) were females. All the patients received an mRNA COVID-19 vaccine. Most patients showed GH within three days after the second or third dose. Among the 82 patients, 22 had been already diagnosed with IgAN or IgA vasculitis (IgAV) before vaccination, and 45 of the 60 undiagnosed patients had a history of abnormal urinary findings. We performed kidney biopsies on 42 of the 60 undiagnosed patients, who were then diagnosed with IgAN (N=41) or IgAV (N=1). Pathological findings demonstrated that chronic inflammation of glomeruli, such as the expansion of mesangial matrix and glomerular sclerosis, is similarly observed in these newly diagnosed patients compared to patients with IgAN unrelated to vaccination. Finally, we evaluated the levels of biomarkers known to be elevated in IgAN at diagnosis during the course of the study and found that they did not increase. Notably, only few cases showed a slight increase in the level of serum creatinine, and no patients progressed to severe renal dysfunction. Conclusion(s): Present prospective study with 82 cases with GH after COVID-19 vaccination have identified their clinical characteristics and outcome. Furthermore, the acute manifestation of vaccine-induced GH may have highlighted the high prevalence of undiagnosed or preclinical IgAN in Japan. No conflict of interestCopyright © 2023
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Introduction: As of June 2021, a total of 120.2 million cases of corona virus disease 2019 (COVID19), with Seven and half lakh COVID 19 related deaths occurred, as estimated by CDC Globally. Vaccination was started by the Government of India to prevent new cases. With the ongoing pandemic, several COVID-19 vaccine agents have received emergency use approval, several adverse effects are being reported with increasing administration of COVID-19 vaccines. Finding(s): Here we describe a case of Henoch Schoenlein purpura, a small vessel vasculitis which is usually seen in children with excellent prognosis in adults, on contrary HSP developed in adult male following COVID-19 vaccination had a rapid deteriorating course. Millions of people are being vaccinated around the world, and thus it is conceivable that people may develop other diseases temporally associated with vaccination but which are unrelated to the vaccine itself. Conclusion(s): Auto Immune Diseases (AID) can be triggered by vaccine but how do they behave when compared to primary AID both in terms of presentation, prognosis and treatment response are not known, thereby we concluded that Henoch Schoenlein purpura can develop post covid vaccination in adults and may have a rapid course and poor prognosis.
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ChAdOx1 nCoV-19 (AZD1222) is a replication-deficient chimpanzee adenovirus vectored vaccine developed by Oxford and AstraZeneca for a disease we all know as Coronavirus, or COVID-19. Ongoing clinical studies reveal that the ChAdOx1 nCoV-19 vaccine has a tolerable safety profile and is effective against symptomatic COVID-19. This vaccine may prove crucial in boosting herd immunity, averting life threatening illness, and relieving the current pandemic. In this mini review, we performed a thorough literature search through PubMed and Google Scholar and reported various case reports associated with complications of the adenovirus-vectored COVID-19 vaccine. Various adverse effects of the ChAdOx1 nCoV-19 vaccine were reported around the globe, which were often serious but rare and developed into life-threatening pathologies such as GBS, thrombocytopenia, demyelinating neuropathies, progressive dementia, cerebral infarction, IgA vasculitis, hemophagocytic lymphohistiocytosis, herpes zoster, cutaneous reactions, and vein thrombosis. These worldwide reported complications, which are usually rare and severe, will aid clinicians in understanding and managing unforeseen situations. There is a need for more research to find out more about these complications and their etiopathogenesis. However, the benefits of these vaccinations for stopping the spread of the outbreak and lowering the fatality rate outweigh the potential risk of the uncommon complications.Copyright © The Author(s) 2023.
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Introduction: Coronavirus disease 2019 (COVID-19) pandemic is ongoing and new variants of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV- 2) are emerging. There is an urgent need for COVID-19 vaccines to control disease outbreaks by herd immunity. Vaccines can trigger immunity as many vaccine-related immunological adverse events have been described. Case Presentation: A case of a 32 years old Filipino female with no known co-morbidity who presented with rashes on bilateral lower extremities one day after receiving her second dose of CoronaVac-Sinovac vaccine. These were non-pruritic nor painful, which appeared to be initially well-defined round erythematous macules, papules, and plaques, mostly raised. It was associated with colicky abdominal pain and inflammatory arthritis affecting the both knees and ankles. She has no vices but known to have allergy with seafood. There were multiple well defined erythematous round to irregularly shaped purpuric macules, papules and plaques, non-blanching, flat and raised, on arms near the antecubital fossa, abdomen and lower extremities. She underwent skin biopsy and direct immunofluorescence showed interface dermatitis with leukocytoclastic vasculitis and IgA +1 vessel wall, and fibrinogen +2 vessel wall, respectively. There was microscopic hematuria and proteinuria. The Urine protein creatinine ratio was normal at 0.193 gm/gm. She was managed as a case of IgA vasculitis and was given moderate dose of steroid (0.5mg per kilogram per day prednisone equivalent) and omeprazole. She was discharged improved with resolution of rashes evident during follow up at the out-patient consultation. Conclusion(s): We report a case of an adult Filipina developing IgA vasculitis following CoronaVac COVID-19 vaccination. She responded well following initiation of steroid therapy. Autoimmune phenomenon following immunization is possible through different mechanisms. These include molecular mimicry, a hyper-stimulated inflammatory state, and autoimmune syndromes induced by adjuvants. While no strategies have been found to prevent autoimmunity following vaccination, it should be emphasized that vaccine recipients should seek medical care for any untoward events following receipt of any immunization.
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Background/Purpose: IgA vasculitis is the most common vasculitis affecting children. Vasculitis can be associated with the inflammatory process following infections, involving single or multiple organs. COVID-19 associated vasculitides have been reported variously, mostly Kawasaki-like features, livedo reticularis and rarely cutaneous small vessels vasculitis. Recently, there have been reports of IgA vasculitis following COVID-19 infection in children, although data among Asians are scarce. Method(s): Case report Results: We herein report a case of a previously healthy 6-year- old Thai boy with history of COVID-19 infection 4 weeks earlier, with only mild upper respiratory tract symptoms treated by a 5 day-course of favipiravir and supportive medication. He presented with rash over both lower limbs with difficulty to bear weight for a week. He denied fever, abdominal pain, nausea, vomiting, or any abnormal urinary symptoms. Physical examination revealed palpable purpura distributed on both lower legs with pain in his left foot and difficulty in bearing weight. His blood pressure was unremarkable for age at 97/67 mmHg. The initial investigations showed complete blood count with white cell count of 8.9 x 103/muL (neutrophils 47.3% and lymphocytes 42.4%), hemoglobin of 13.6 g/dL, which had no anemia for his age and platelet count of 297 x 103/muL. His urinalysis showed 2-3 red blood cells and 0-1 white blood cells per high power field without proteinuria and normal renal function. The erythrocyte sediment rate was 11 mm/hr and c-reactive protein was 3.9 mg/L, which were in normal range. He was diagnosed as IgA vasculitis and non-steroidal anti-inflammatory drug was prescribed to alleviate arthralgia of left foot. A week later, he revisited due to pain and swelling at his left scrotum. He was diagnosed as orchitis, one of the clinical manifestations of IgA vasculitis that can occur in boys. He had ongoing palpable purpura on the legs but pain at the left foot subsided. He then received oral prednisolone for the indication of orchitis at the dosage of 1 mg/kg/day with subsequent tapering for total duration of 3 weeks. All of his symptoms completely resolved. Conclusion(s): We present the interesting case of a Thai boy clinically diagnosed with IgA vasculitis following COVID-19 infection, having the clinical manifestations of palpable purpura, arthralgia, and orchitis. There are very limited data about post COVID-19- associated IgA vasculitis in children, especially in the Asian population. We would like to highlight this condition for physicians and to raise the awareness in the COVID-19 era.
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Background: Autoimmune inflammatory rheumatic diseases (AIRD) were associated with an increased risk for COVID-19 infection, worse clinical outcomes, and COVID-19- related deaths. Vaccines carry the potential benefit of reducing disease transmission and disease severity. Issues on vaccine safety, trigger of an autoimmune reaction or disease flares has been a long issue. Thus this study describes the COVID-19 vaccination status and adverse events following SARS-COV 2 vaccine in a tertiary hospital in Manila, Philippines. Method(s): This retrospective cross-sectional study included patients diagnosed with AIRD and seen in the clinics over 12 months (March 2021-March 2022).We collected data from patients' clinic records and analyzed the clinicodemographic profile, vaccination status, adverse events and development of new onset autoimmune reaction and disease flares post vaccination and those who developed COVID-19 infection even after vaccination. Result(s): There were 204 respondents included in this study: 174 (85%) were female and 30 (14%) were male;63.2 % with SLE, 10.8% rheumatoid arthritis, 8.3% psoriatic arthritis, 6.4% ankylosing spondylitis and other systemic autoimmune disease. Others included were scleroderma and inflammatory arthritis and myopathies. The median age is 38.4 years. 100% of the respondents were fully vaccinated;87 (42.6%) received inactivated vaccine, 63 (30.8%) viral vector vaccine and 54 (26.4%) mRNA vaccine. 133 (65.2%) reported no adverse events while 71 (34.8%) experienced constitutional symptoms, musculoskeletal, cutaneous, respiratory, gastrointestinal and cardiovascular symptoms after vaccination. There were 9 (4.4%) of the respondents who developed new onset autoimmune reaction and disease flares 7-28 days post vaccination;1 patient had new onset amyopathic dermatomyositis;3 SLE patients developed severe hematologic, cutaneous and renal flare;2 rheumatoid arthritis, 1 spondyloarthritis, and 1 inflammatory arthritis patients in long remission had disease flares and 1 patient with henoch schonlein purpura developed flare. Twenty two (10.8%)of the respondents developed COVID-19 infection after 30-120 days post vaccination;19 (86.4%) had mild symptoms, 2 (9.1%) moderate infection and 1 (4.5%) had severe infection and all of the patients recovered. Conclusion(s): This study showed that patients with AIRD experienced varied organ system adverse events following vaccination ranging from mild side effects, disease flares and new onset autoimmune reactions. The development of an autoimmune reaction following SARS-COV 2 vaccination is of scientific and public importance and still needs to be investigated. Even after a complete vaccination, some patients still had COVID-19 infection, however preventing them from worse complications, hospitalization and mortality.
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Introduction: Vaccination against COVID-19 is essential, however an immunological flare is a potential rare complication resulting in glomerulonephritis with IgA deposits in the mesangium. We present a case of a patient who developed IgA nephropathy postvaccination. Case Description: 48-year-old woman with a past medical history of Leukocytoclastic vasculitis and hypertension presented to the Emergency Department with fatigue, nausea, epigastric pain, foamy urine and a diffuse erythematous purpuric rash within days of receiving the SARS CoV-2 vaccination. Her creatinine was 1.92 mg/dL and urinalysis showed 3+blood and 30 mg/dL of protein. COVID-19 testing was negative. Protein/ creatinine ratio was 2.1 g/g and urine microscopy showed dysmorphic red blood cells. Serologies for HIV, Hepatitis B and C were negative. Further testing revealed negative ANA, normal ASO titer, absent cryoglobulins, rheumatoid factor < 20, C3 158 (nl) and C4 35 (nl). However, IgA level was elevated at 462 mg/dL (reference 70-312 mg/dL). Patient was started on prednisone at a dose of 1mg/kg with a presumptive diagnosis of IgA vasculitis/ HSP. Subsequent skin biopsy was consistent with leukocytoclastic vasculitis while kidney biopsy showed glomerular deposition of IgA and endocapillary hypercellularity. At one month follow-up with nephrology, prednisone taper was started because of good clinical response and partial remission with UPCR reduction to 1 g/g. Prednisone was gradually tapered over the next 2 months. At her 3 month follow-up she was found to be in complete remission with a UPCR 0.2 g/g and her creatinine was 0.83 mg/dL Discussion: We present a case of IgA nephropathy post-SARS CoV-2 mRNA vaccination. It has been speculated that the mRNA lipid nanoparticle-encapsulated platform contained within the mRNA vaccine produces such a robust CD4 and CD8 T-cell response that pro-inflammatory cytokines activate this immune complex associated glomerular disease. In conclusion, SARS CoV-2 vaccination may potentially trigger IgA nephropathy in predisposed patients. Steroid therapy may be efficacious in managing this rare complication.
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Introduction: Infection-related IgA glomerulonephritis with large paramesangial immune deposits appearing like intraluminal cryo deposits have occasionally been described in literature temporally associated with Staphylococcus infection. COVID-19 associated glomerular disease is classically known to manifest as collapsing glomerulopathy, although other types have been reported. Case Description: We present a case of a 76-year old Hispanic female with a history of type 2 diabetes, hypertension and a 2-month old history of COVID-19 pneumonia who recently presented with acute kidney injury, dark urine, shortness of breath and leg edema. 2 weeks prior, she had presented with a history of purpuric rash. Urinalysis had shown hematuria and proteinuria, and a skin biopsy showed IgA vasculitis. Blood culture for Staphylococcus was negative. A kidney biopsy now showed IgA glomerulonephritis with focal crescents along with intraluminal pseudothrombi (cryo-plugs) positive for IgA on immunofluorescence. Electron microscopy showed intraluminal occlusive electron-dense deposits. Serum cryoglobulin was negative. The patient was treated with steroids and oral cyclophosphamide, and she responded significantly to treatment. Discussion(s): Our case shows a unique glomerular manifestation of COVID-19 infection in the form of IgA vasculitis with intraluminal cryo-like features, not previously described in literature.
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Introduction: A 24 year old male presented with rash, gastrointestinal bleeding and nephrotic syndrome one week after first COVID vaccination. Renal biopsy revealed crescentic IgA nephritis. He was treated steroids and responded clinically but continues to have proteinuria. Case Description: A 24 year old male with history of cerebral palsy presented with a vasculitic rash, hematochezia and edema. He had no prior history of renal or autoimmune disease. Symptoms developed one week after first dose of Moderna mRNA-1273 vaccine. Serum albumin was 1.6 g/dL and urine protein-creatinine ratio 8.5. Endoscopy showed esophagitis and small bowel ulcerations. Renal biopsy showed focally crescentic and necrotizing proliferative glomerulitis with IgA dominant deposits consistent with IgA vasculitis with renal involvement. He received pulse dose solumedrol followed by prednisone taper. Cyclophosphamide was initiated but then stopped due to cytopenias. After several weeks GI bleeding resolved spontaneously and proteinuria improved but remained in the nephrotic range. The course was further complicated by positive COVID testing prior to discharge-he was asymptomatic and received sotrovimab. On followup three months later the patient's edema resolved and serum albumin normalized. He continues to have subnephrotic range proteinuria with 2.3 grams/24 hours and active urinary sediment with dysmorphic red cells. He remains on steroid taper with plan for repeat renal biopsy to inform decisions regarding further immune suppression. He has not been rechallenged with COVID vaccine. Discussion(s): Rare associations between COVID vaccination and both de-novo and relapsing glomerular lesions, including minimal change disease, IgA nephropathy, ANCA and anti GBM glomerulonephritis, have been reported. COVID infection itself has been associated with an FSGS type lesion termed COVID-associated nephropathy (COVAN). As highlighted by this case, unvaccinated or partially vaccinated patients are at increased risk for COVID infection. Thus even in those with known glomerular disease, vaccination should still be recommended. Studies are needed to determine if patients with off-target glomerulopathies can be safely rechallenged with COVID vaccine, whether the course of COVID vaccine-associated disease glomerular lesions mimic the primary glomerular disease and how to optimally manage these patients.
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Introduction: IgA vasculitis (IgAV) is a common diagnosis in children and includes purpura, and/or petechiae (without thrombocytopenia or coagulopathy) with at least one of the following: abdominal pain, joint pain, AKI, hematuria, proteinuria, or evidence of IgA deposition. Many cases are preceded by upper respiratory tract infections, including COVID-19. The incidence of cerebral venous sinus thrombosis (CVST) in the pediatric population is low (0.6/100,000 per year). We present a case of a 5 year old boy with IgA vasculitis and COVID-19 infection found to have CVST. Case Description: A previously healthy 5 year old boy transferred to our institution with two weeks of intermittent, severe abdominal pain in the setting of COVID-19 infection with new-onset hematochezia, hypertension, and tachycardia. Abdominal ultrasound, abdominal x-ray, chest x-ray, ANA, C3, C4, ANCA, creatinine, electrolytes, and coagulation factors were normal. Urinalysis was significant for hematuria and a urine protein-to-creatinine ratio (UPC) of 2.02 mg/mg. Purpuric and petechial rash appeared the day after admission. UPC trended up to 4.82 mg/mg and a renal biopsy confirmed the diagnosis of IgA nephropathy. Patient was treated with 30mg/kg/day Solu-Medrol for three days and discharged home on 2mg/kg/day prednisolone daily. He was readmitted two days later with severe left frontal headache. UPC was worse at 5.98 mg/mg and mycophenolic mofetil (MMF) was initiated. Imaging revealed an occlusive thrombus of the left transverse sinus with nonocclusive thrombi in the distal portion of the left lateral sinus and posterior superior sagittal sinus. He started 21mg Lovenox twice daily and had minimal residual thrombosis after three months. His UPC peaked at 20.73 mg/mg and eventually normalized with high-dose steroids, Enalapril, and MMF. Discussion(s): This is the first case, to our knowledge, of CVST in a patient with IgAV associated with COVID-19 infection. Multiple case reports of IgA vasculitis associated with COVID-19 infection have been published in the past two years, and this case may support a more careful approach when it comes to screening for pro-coagulation risk factors.
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Introduction: The systemic vasculitides are classified based on the size of the vessels involved and ANCA vasculitis is the most common to involve the small vessels of the kidney. Granulomatous tubulointerstitial nephritis (TIN) without glomerular involvement has been described in ANCA vasculitis but is not commonly reported with medium vessel vasculitis. We present a case of necrotizing granulomatous TIN associated with systemic polyarteritis nodosa (PAN). Case Description: A 71-year-old female was admitted with a two-day history of purpuric lower extremity rash, abdominal pain, and AKI (serum creatinine 1.8 mg/dL from baseline 0.6 mg/dL). There was initially concern for IgA vasculitis and prednisone was started. A skin biopsy revealed leukocytoclastic vasculitis but negative IF. A kidney biopsy was notable for necrotizing granulomatous TIN but otherwise unremarkable appearance of the glomeruli. As her AKI and overall clinical status worsened, she was started on HD. Due to worsening liver function, a liver biopsy was done showing neutrophilic inflammation but no granulomas. Rheumatologic workup notable only for ANA 1:360 (normal complements, negative ANCA, MPO, PR3, RF, cryoglobulins). Infectious workup (TB, fungal, and parasitic) was unremarkable. There was neither NSAID nor antibiotic exposure prior to the kidney biopsy. Only home medication was losartan. She received dose 2 of Moderna COVID-19 vaccination 4 weeks prior. Her kidney function improved and dialysis was stopped, however, she developed a new foot drop. A sural nerve biopsy showed necrotizing vasculitis of a medium sized vessel. The clinical picture was felt to be most consistent with PAN. Her steroid dose was increased and she started Cytoxan per CYCLOPS protocol with subsequent clinical improvement. Discussion(s): There are few prior reports of isolated TIN associated with PAN and there are often confounding exposures that can potentially explain AIN. In the present case, the early histologic diagnosis of TIN in the absence of known exposures suggests the TIN is likely associated with, and potentially secondary to, the concurrent vasculitic process. Clinically, her presentation may represent a small and medium vessel vasculitis overlap syndrome as her pathology was not entirely consistent with an ANCA-negative pauci-immune vasculitis nor PAN. Alternatively, the TIN may simply be a response to the systemic inflammatory condition.
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Introduction: Alport syndrome is a rare inherited disorder manifested by persistent microscopic hematuria, nephritis, sensorineural deafness, and ocular abnormalities. IgA nephropathy has been reported in a small percentage of patients with Alport syndrome, however, IgA vasculitis nephritis (IgA-VN) has not been reported. We report a rare case of Alport syndrome with crescentic IgA-VN. Case Description: A 48-year-old male with a history of deep vein thrombosis (on warfarin) was followed by nephrology clinic for chronic kidney disease (CKD). Due to concerns of risks associated with holding warfarin, genetic testing was done in lieu of kidney biopsy which was positive for a hemizygous pathogenic mutation in the X-linked COL4A5 gene for Alport Syndrome. He did not have any sensorineural deafness, ocular abnormalities, or a family history of kidney disease. He was admitted to the hospital for acute kidney injury (creatinine 14.6 mg/dL from a baseline of 2 mg/dL), nephrotic range proteinuria (microalbumin/creatinine ratio: 3,689 mg/g, normal < 200 mg/g) and microscopic hematuria. A full serological workup was unremarkable. He developed newonset bilateral lower extremity purpura which was biopsied showing leukocytoclastic IgA Vasculitis. Kidney biopsy showed IgA-dominant glomerulonephritis with 70% interstitial fibrosis and tubular atrophy, 80% crescents, and no electron microscopy findings suggestive of Alport Syndrome. Four days after the kidney biopsy, he was started on dialysis and immunosuppression (IV Methylprednisolone and Cyclophosphamide) after ruling out infection. He was discharged with a plan to continue immunosuppression and dialysis as an outpatient. Unfortunately, he developed COVID-19 prompting a delay in further immunosuppressive therapy. Discussion(s): Although extremely rare, mutations in the COL4A3, COL4A4, and COL4A5 genes have been implicated in cases of familial IgA Nephropathy and Alport Syndrome. We report a novel patient with IgA-VN and COL4A5 variant. It is unclear whether his underlying COL4A5 variant contributed to his severe presentation with IgAVN. Genome-wide association studies in patients with coexisting pathologies are needed to discover both diseases' possible common genetic connection.
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Upon COVID-19 infection, age-specific mortality rates in RADs patients notably began from 35 years old, while in the uninfected population, it was from 55. COVID-19 associated rheumatic signs and symptoms are myalgia, fatigue, Kawasaki-like signs, and skin rashes mimicking vasculitides and pernio (chilblains) like lesions. So a variety of rheumatic diseases may mimic or be mimicked by COVID-19. Rheumatologic Treatments During COVID-19 Epidemic: Prednisone caused an increased hospitalization rate, significantly when the dose exceeded 10 mg per day. It is reasonable to reduce glucocorticoids gradually to 5 - 7.5 mg/day, but discontinuation during the pandemic is not recommended. Conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) reduce the risk of COVID-19 infection and the cytokine storm emerging in severe cases. Colchicine has reduced the mortality of COVID- 19 patients and the number of severe cases. Tapering or even discontinuing csDMARDs is suggested to recover immunity in severe cases, which may help rapidly eliminate the virus. Hydroxychloroquine is likely to increase survival in SLE patients, and it is not advisable to be discarded. Biologic or targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs) may help reduce inflammatory cytokine storm under COVID-19 attack. Compared with RADs patients treated with CD20 monoclonal antibody rituximab or IL-17A antagonist secukinumab, patients receiving tumor necrosis factor (TNF) inhibitors etanercept and alemtuzumab or IL- 6 receptor antagonist tocilizumab may experience milder course. Applicable Laboratory Indicators: Elevation of ESR, CRP, ferritin, interleukin 6, and creatine kinase can be seen in COVID-19 and various rheumatic diseases. RADs related autoantibodies may present among non-RAD severe COVID- 19 cases. COVID-19 as a Risk Factor for Rheumatologic Diseases: Cases of Small vessel cardiac vasculitis/endothelium, immunoglobulin A (IgA) vasculitis in patients with Crohn disease, cutaneous vasculitis-like lesions, systemic arterial and venous thromboembolism including cryptogenic strokes and other vasculopathy features, systemic rheumatic diseases such as SLE, inflammatory arthritis, GCA, inflammatory myopathies, APS, Sjogren's syndrome, ANCA-associated vasculitides, seropositive rheumatoid arthritis, and Virus-associated or reactive arthritis and Crystal-related arthritis due to gout or calcium pyrophosphate disease has been reported. COVID-19, in the acute phase, may cause cytokine storm and severe inflammatory response;and in the chronic phase, patients become susceptible to autoinflammatory and autoimmune diseases. If a patient has signs and symptoms of rheumatic diseases after developing COVID-19, do not attribute these complaints entirely to COVID-19;consider starting a real dangerous rheumatic disorder.